Leiner Syndrome

Severe seborrheic dermatitis with systemic infection, diarrhea, and central nervous system deficiency as a consequence of complement C5 deficiency (acquired deficiency in most patients).

Complement C5 Deficiency; Dermatitis Exfoliativa Generalisata; Desquamative Erythroderma in Infants; Eczema Universale Seborrhoeicum; Erythrodermia Desquamativa of Leiner; Erythrodermia Desquamativa in Infants; Erythroderma Desquamativum; Erythrodermic Seborrheic Dermatitis in the Trianon of Life; Exfoliative Dermatitis; Seborrhoic Diathesis in Infants; Leiner-Moussous Disease.

First described in 1908 by Karl Leiner, an Austrian pediatrician.

Prevalent in infant females. Breast-fed infants are most frequently affected.

Not a genetic disorder in most patients. Gene map locus of hereditary complement C5 deficiency is 9q34.1.

Leiner disease includes a heterogeneous group of disorders. A common feature of these disorders is temporary or permanent (hereditary forms) plasma deficiency in complement C5 activity resulting in decrease of opsonic properties of plasma (deficiency in phagocytosis).

Clinical picture. Deficiency in complement C5 activity in plasma.

Syndrome characterized by desquamation (severe generalized seborrheic dermatitis), recurrent local and systemic infection, severe diarrhea, marked wasting, central nervous system deficiency, and failure to thrive. It begins as seborrheic eczematoid lesions of the scalp and face or the gluteal region, eventually spreading to other areas. Keratitis and corneal ulcers may occur. Caused by complement C5 deficiency in infancy; rapid onset in second to fourth month of life.

Elective anesthesia and surgery should be postponed until skin covering is restored in relevant areas.

Patients with Leiner disease are prone to severe bacterial contamination as a consequence of their extensive skin lesions and deficiency in phagocytosis. Large spectrum antibioprophylaxis should be considered. Because of extended skin lesions, there is a danger of systemic toxicity with topical agents (especially disinfectants and skin preparation solutions).

Use topical agents with extreme care because of increase systemic absorption and toxicity. Avoid any solution containing potentially toxic components, either topical or systemic. Some patients receive high-dose corticosteroid in the acute phase, so intraoperative supplementation will be needed.

Other disorders with erythroderma (some of which are included in the general term of Leiner disease), including the following:

Atopic Eczema: Later onset, familial context of atopy.

Infantile Seborrheic Dermatitis: “Cradle cap,” usually self-limiting, subsiding by age 6 months; no complement C5 deficiency; cured by topical cortisone.

Ichthyosiform Erythroderma: Autosomal dominant; bullous ichthyosis.

Netherton Syndrome: Recessive skin atopy disorder, bamboo hair, mostly in females.

Immunodeficiency Syndromes: Group of syndromes that have in common a dysfunction of the immune system. They are divided into T-cell system deficiency or B-cell deficiency. For instance, Idiopathic Thrombocytopenic Purpura (ITP), X-linked Agammaglobulinemia, Severe Combined Immunodeficiency, Nezelof Syndrome, and Wiskott-Aldrich Syndrome are all considered immunodeficiency syndromes.